Feminizing adrenocortical tumor: steroid hormone response to ketoconazole.
A 58-yr-old man presented with gynecomastia and elevated serum estrogens. The diagnosis of an estrogen-secreting adrenal tumor was made based upon the finding of a 4-cm left adrenal mass, elevated levels of estradiol in peripheral and left adrenal venous blood, and increased urinary 17-ketosteroids. In addition to marked elevations in estradiol and 17-ketosteroids there was an increased baseline level of 11-deoxycorticosterone and a slightly decreased level of 18-hydroxycorticosterone, suggesting the possibility of impaired P450c11 activity. The effect of ketoconazole administration (600 mg/day) for 4 weeks was studied. Urinary free cortisol and 17-ketosteroid excretion and serum testosterone levels fell acutely (1 week). Serum estradiol levels decreased gradually over the 4-week course. Plasma aldosterone levels were essentially unaltered and 18-hydroxcorticosterone levels fell gradually, but there were marked increases in 11-deoxycorticosterone and corticosterone. Coincident with the increase in 11-deoxycorticosterone there was an increase in blood pressure and a transient fall in serum potassium. We conclude that ketoconazole administration may result in a hypermineralocorticoid state. Therefore, the usefulness of ketoconazole therapy for steroid hormone-producing neoplasms will depend upon the individual tumor's steroidogenic profile.
Saadi
H F
HF
Division of Endocrinology, Case Western Reserve University, School of Medicine, Cleveland, Ohio 44106.
Bravo
E L
EL
Aron
D C
DC
D015068:17-Ketosteroids / Q000378:metabolism; D000306:Adrenal Cortex Neoplasms / Q000150:complications / Q000188:drug therapy* / Q000378:metabolism; D000450:Aldosterone / Q000378:metabolism; D013734:Androgen-Insensitivity Syndrome / Q000209:etiology* / Q000378:metabolism; D003345:Corticosterone / Q000378:metabolism; D003900:Desoxycorticosterone / Q000378:metabolism; D004958:Estradiol / Q000378:metabolism; D004967:Estrogens / Q000378:metabolism; D006801:Humans; D006854:Hydrocortisone / Q000378:metabolism; D007654:Ketoconazole / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D013739:Testosterone / Q000378:metabolism
D015068:17-Ketosteroids; D004967:Estrogens; D013739:Testosterone; D003900:Desoxycorticosterone; D000450:Aldosterone; D004958:Estradiol; D007654:Ketoconazole; D003345:Corticosterone; D006854:Hydrocortisone